What is hepatoblastoma?

Hepatoblastoma is a very rare cancerous tumor that starts in the liver. The liver is one of the largest organs in the body. The primary functions of the liver include filtering and storing blood. The liver consists of right and left lobes.

This disease primarily affects children from infancy to about 3 years of age. Hepatoblastoma cancer cells can spread (metastasize) to other areas of the body, but this is rare.

Anatomy of the liver

The liver is located in the upper right-hand portion of the abdominal cavity, beneath the diaphragm and on top of the stomach, right kidney, and intestines. Shaped like a cone, the liver is a dark reddish-brown organ that weighs about three pounds.

The liver consists of two main lobes, both of which are made up of thousands of lobules. These lobules are connected to small ducts that connect with larger ducts to ultimately form the hepatic duct. The hepatic duct transports the bile produced by the liver cells to the gallbladder and duodenum (the first part of the small intestine). The liver regulates most chemical levels in the blood, excretes a product called "bile," which makes clotting factors that keep the body from bleeding when injured, and helps remove waste products from the body.

What causes hepatoblastoma?

Although the exact cause of liver cancer is unknown, there are a number of genetic conditions that are associated with an increased risk for developing hepatoblastoma, including Beckwith-Wiedemann syndrome, Wilson disease, porphyria cutanea tarda, and familial adenomatous polyposis. Other genetic conditions associated with liver cancer include several inborn errors of metabolism such as tyrosinemia, glycogen storage diseases, and alpha1-antitrypsin deficiency.

Children who are exposed to hepatitis B or hepatitis C infection at an early age, or those who have biliary atresia, are also at increased risk for developing hepatocellular carcinoma. Some hepatoblastomas have genetic alterations in tumor suppressor genes, which would explain the uncontrolled cell growth.

What are the symptoms of hepatoblastoma?

The following are the most common symptoms of hepatoblastoma. However, each child may experience symptoms differently. Symptoms may vary depending on the size of the tumor and the presence and location of metastases. Symptoms may include:

A large abdominal mass, or swollen abdomen

Weight loss, decreased appetite

Early puberty in boys

Abdominal pain

Nausea and vomiting

Jaundice (yellowing of the eyes and skin)

Fever

Itching skin

Enlarged veins on the belly that can be seen through the skin

The symptoms of hepatoblastoma may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.

How is hepatoblastoma diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for hepatoblastoma may include:

Biopsy - a sample of tissue is removed from the tumor and examined under a microscope.

Complete blood count (CBC) - a measurement of size, number, and maturity of different blood cells in a specific volume of blood.

Computed tomography scan (Also called a CT or CAT scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce horizontal, or axial, images (often called slices) of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.

Magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.

Ultrasound (Also called sonography) - a diagnostic imaging technique which uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.

Alpha-fetoprotein (AFP) test - alpha-fetoprotein (AFP) levels in the blood can be used to diagnose and follow response to treatment.

What are the different stages of childhood liver cancer?

Staging is the process of determining whether cancer has spread and, if so, how far. There are various staging symptoms that are used for hepatoblastoma. Always consult your child's physician for information on staging. One method of staging hepatoblastoma is the following:

Stage I - usually a tumor that in only in the liver and can be completely removed with surgery.

Stage II - usually a tumor that can mostly be removed by surgery but very small amounts of the cancer are left in the liver.

Stage III - usually a tumor that cannot be completely removed or cancer cells are found in nearby lymph nodes.

Stage IV - cancer that has spread (metastasized) to other parts of the body.

Recurrent - the disease has returned after it has been treated. It may come back in the liver or in another part of the body.

Treatment for hepatoblastoma

Specific treatment for hepatoblastoma will be determined by your child's physician based on:

As with any cancer, prognosis and long-term survival can vary greatly from child to child. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a child diagnosed with hepatoblastoma. New methods are continually being discovered to improve treatment and to decrease side effects.

For more information or to schedule an appointment, call 314.454.5437 or 800.678.5437 or email us.